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Overview
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PDHA1 is a protein encoded by the PDHA1 gene which is approximately 43, 3 kDa. PDHA1 is localised to the mitochondrion matrix. It is involved in the citrate cycle, pyruvate metabolism, glucose metabolism and carbon metabolism. PDHA1 is one of three PDH complexes. This complex is a nuclear-encoded mitochondrial multienzyme complex that catalyses the overall conversion of pyruvate to acetyl-CoA and CO2, and provides the primary link between glycolysis and the tricarboxylic acid cycle. PDHA1 is expressed ubiquitously. Mutations in the PDHA1 gene result in pyruvate dehydrogenase E1-alpha deficiency, Leigh syndrome with leukodystrophy and Coffin-Lowry syndrome. STJ95006 was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. This polyclonal antibody detects endogenous levels of PDHA1 protein.
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